Diffuse Leiomyomatosis of the Esophagus

Abstract

Background: Leiomyomas are rare esophagus neoplasms. They are usually solitary, and the diffuse lesion is extremely rare. Case Report: A 19-year-old male presented with a 3-year history of occasional dysphagia and postprandial regurgitation. The chest radiographs showed a huge mass in the posterior mediastinum. Barium esophagograms showed narrowing of the middle third esophagus with proximal dilatation. The fibroesophagoscopy demonstrated multiple submucosal nodules below a level 22 cm from the incisor and covered with intact mucosa. CT scans of the chest showed a long segment of circumferential soft tissue in the posterior mediastinum which encircled and involved the upper two thirds of the esophagus. An intrathoracic esophagectomy with cervical esophagogastrostomy via the substernal route was performed. Grossly, multiple confluent myomatous nodules circumferentially involved the upper and middle third of the esophagus. Histologic findings showed diffuse leiomyomatosis of the esophagus. Conclusion: Esophageal leiomyomatosis should be considered in a young patient with long-standing dysphagia in whom smooth, tapered esophageal narrowing on barium study and circumferential esophageal wall thickening on CT scan are seen. An esophagectomy combined with a reconstruction procedure is indicated.

Diffuse esophageal leiomyomatosis

“…The esophagus accounts for less than 10% of all gastrointestinal leiomyomas, and leiomyomas account for only 0.4% of all esophageal tumors 2 . Esophagectomy and reconstruction has replaced myotomy as the preferred treatment for diffuse esophageal leiomyomatosis due to its anatomical location in the lower (53%) and middle (35%) thirds of the esophagus 2–5 . At 12‐month follow‐up, our patient was well with no recurrence of dysphagia.…”

Multiple Esophageal Leiomyoma Presenting with Clinical Dysphagia from Mechanical Obstruction and Motility Disorder

“…It is the most common benign tumor of the esophagus accounting for 0.4% of esophageal neoplasms and approximately two-thirds of benign esophageal tumors. About 90% of them are reported to be solitary and intramural lesions, and multiple or diffuse lesions seem to be very rare [7]. These tumors originate from the smooth muscle in the muscular layer of the esophagus.…”

Multiple solitary leiomyomas in the esophagus: Report of a case

“…3,9 Leiomyomas are usually solitary, rounded, and welldemarcated masses, and multiple leiomyomas (more than three) are extremely rare. 3,4 In a review of 838 cases, Seremetis et al 3 emphasized that multiple solitary leiomyomas should not be con- fused with diffuse leiomyomatosis.…”